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A tracheoesophageal fistula (TEF) is an abnormal connection (fistula) between the esophagus and the trachea. TEF is a common congenital abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy.
Congenital TEF can arise due to failed fusion of the tracheoesophageal ridges during the third week of embryological development.[1]
A fistula, from the Latin meaning ‘a pipe,’ is an abnormal connection running either between two tubes or between a tube and a surface. In tracheo-esophageal fistula it runs between the trachea and the esophagus. This connection may or may not have a central cavity; if it does, then food within the esophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the esophagus.
Babies with TEF or esophageal atresia are unable to feed properly. Once diagnosed, prompt surgery is required to allow the baby to take in food. Many TEF children have few problems after surgery, however a number develop feeding difficulties and chest problems. Some TEF babies are also born with other abnormalities, most commonly those described in VACTERL association – a group of anomalies which often occur together, including heart, kidney and limb deformities.
Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location,[2][3] however, various pediatric surgical publications have attempted a classification system comprised of the below specified types.
Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do.
The letter codes are usually associated with the system used by Gross,[6] while number codes are usually associated with Vogt.[7]
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